BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare t...BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.展开更多
Objective: Primary lymphoma of bone (PLB) is a rare malignant bone tumor often presenting in the fifth-sixth decades involving appendicular long bones. Published radiological findings indicate that PLB typically prese...Objective: Primary lymphoma of bone (PLB) is a rare malignant bone tumor often presenting in the fifth-sixth decades involving appendicular long bones. Published radiological findings indicate that PLB typically presents as a moth-eaten osteolytic lesion with periosteal reaction, while MRI commonly demonstrates marrow infiltration with extraosseous extension. Given rarity and variable appearances, PLB may not be primarily considered prior to biopsy. Our objective was to evaluate preoperative imaging findings in effort to increase awareness and improve a perceived deficiency in preoperative diagnosis. Materials and Methods: Following IRB approval, retrospective review identified 60 patients with newly diagnosed bone lesions proven to represent PLB in accordance with WHO definition. Preoperative radiographs (n = 46), MRI (n = 33) and PET (n = 37) were independently reviewed by two radiologists. At radiography, lesions were classified: purely lytic, mixed, purely sclerotic, or occult;lytic lesions were graded utilizing Lodwick’s classification. At MRI, lesions were defined as focal or infiltrative and the presence or absence of extraosseous disease was recorded. Extraosseous masses were defined as small (1 cm) and subjectively correlated with degree of cortical destruction. At PET, lesions were recorded as FDG-avid or not. Primary radiograph reports when available (n = 33) were reviewed and exact wording of differential considerations was recorded. Results: Radiographs demonstrated mixed (n = 22), lytic (n = 15), and sclerotic (n = 8) appearances;one radiographically occult lesion was seen by MRI. Lytic lesions were graded: IB (n = 3), IC (n = 5), II (n = 4), and III (n = 3);none were IA. At MRI, 30 lesions were infiltrative and 3 were focal;11 were not associated with extraosseous extension, while 22 showed bony disease with small (n = 7) or large (n = 15) soft tissue mass. Of large masses, 13 demonstrated minimal cortical destruction. At PET, 36 demonstrated FDG uptake;one study was technically limited. Review of reports found that only 5 included “lymphoma” as a diagnostic consideration. Conclusion: Contrary to most published data, we suggest that PLB typically demonstrates some degree of osteosclerosis, often a mixed pattern of sclerosis and lucency;purely lytic lesions may be less common. Similar to existing reports, MRI commonly demonstrates marrow infiltration with extraosseous extension of disease, typically a large soft tissue mass with disproportionate (minimal) cortical destruction. Familiarity with these findings should improve preoperative consideration of PLB in the appropriate clinical scenario when a new osteoblastic lesion is identified.展开更多
Primary mediastinal neuroendocrine tumors are a rare malignancy that accounts for < 10% of all mediastinal tumors. The case presented here involves a 52-yearold man who had been suffering for 3 mo from chronic coug...Primary mediastinal neuroendocrine tumors are a rare malignancy that accounts for < 10% of all mediastinal tumors. The case presented here involves a 52-yearold man who had been suffering for 3 mo from chronic cough, anorexia and substantial weight loss, as well as 2 wk of jaundice prior to his admission. A computed tomography scan showed a 4.3 cm × 6.6 cm mediastinal mass with multiple liver nodules scattered along both hepatic lobes. Endoscopic ultrasound showed a large heterogeneous hypoechoic mass at the mediastinum with multiple target-like nodules in the liver. Fine-needle aspiration specimens revealed numerous, small, round cells with hyperchromatic nuclei, scarce cytoplasm, and frequent mitotic features. Immunohistochemical study revealed positive results for AE1/AE3, CD56 and chromogranin A, with negative findings for synaptophysin, CK20, vimentin, CK8/18 and CD45. The patient was subsequently diagnosed with a poorly differentiated neuroendocrine carcinoma, small cell type. A bone marrow biopsy also revealed extensive involvement by the carcinoma.展开更多
Primary non-Hodgkin’s lymphoma of the bone (PLB) is extremely rare in the pediatric population with less than 100 cases reported in the English literature. Most commonly, patients present with atraumatic bone pain an...Primary non-Hodgkin’s lymphoma of the bone (PLB) is extremely rare in the pediatric population with less than 100 cases reported in the English literature. Most commonly, patients present with atraumatic bone pain and grossly normal radiographic findings. PLB is in the histopathological class of “small round cell tumors of bone”, as with most common bone tumors. The diagnosis is confirmed by immunohistochemical or flow cytometry based detection of tumor-specific proteins. We present a case of stage IV PLB of B-lymphoblastic type with an excellent response to chemotherapy to increase awareness among general pediatricians and pathologists about the importance of making the correct diagnosis, given the excellent prognosis for this disease.展开更多
Introduction: Diffuse large B-cell lymphoma (DLBCL), not otherwise specified, is a large B-cell lymphoma with a diffuse growth pattern and aggressive clinical course. It is divided in subgroups according to its morpho...Introduction: Diffuse large B-cell lymphoma (DLBCL), not otherwise specified, is a large B-cell lymphoma with a diffuse growth pattern and aggressive clinical course. It is divided in subgroups according to its morphology, immunophenotype, and primary site. Dissemination to bone marrow occurs in 11% to 35% of cases and can be of concordant or discordant morphology. Objective: To examine the association, the type of bone marrow involvement in relation to the primary site, morphology, immunohistochemistry of DLBCLs and to determine the cases of Epstein-Barr virus positive DLBCLs. Materials and Methods: We reviewed lymph node and extranodal biopsies as well as the respective bone marrow biopsies in all cases of DLBCL diagnosed in the Hospital General de México during the period from 2002 to 2010. We used immunohystochemistry for immunophenotype identification (Hans’s algorithm) and an in-situ hybridization technique to detect presence of Epstein Barr encoded RNA (EBER). Results: We included 108 patients with a mean age of 51.9 years, 59 (55%) were men. DLBCL involved lymph nodes in 60% of cases and palatine tonsils in 13%. The centroblastic variant predominated (80%) and 58% originated from activated B-cells. Infiltration of bone marrow was present in 30% of cases and was discordant in 55% of these cases. Correlation between morphology and bone marrow infiltration was statistically significant (P = 0.0003). Presence of Epstein-Barr virus was demonstrated in 15% of patients older than 50 years. Conclusions: Dissemination to bone marrow occurred in 30% of cases and discordant involvement was most common. DLBCL originating from activated B-lymphocytes predominated and the most common extranodal sites were palatine tonsils, suggesting that our population has a clinical behavior similar to Asiatic populations.展开更多
目的探讨血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T cell lymphoma,AITL)侵犯骨髓的病理特征。方法回顾性分析32例AITL侵犯骨髓的临床病理特征,采用免疫组化EnVision法和流式细胞术检测AITL相关免疫标记,通过T系基因重排分析T...目的探讨血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T cell lymphoma,AITL)侵犯骨髓的病理特征。方法回顾性分析32例AITL侵犯骨髓的临床病理特征,采用免疫组化EnVision法和流式细胞术检测AITL相关免疫标记,通过T系基因重排分析T细胞克隆性。结果肿瘤细胞浸润模式主要以结节状(20/32,62.5%)、间质性或小簇状(10/32,31.3%)为主,结节成分较杂,可呈“肉芽肿样改变”;肿瘤细胞主要为小至中等大小淋巴细胞,异型不明显,少数病例可出现明显浆细胞增生。19例行免疫组化染色,CD4阳性T细胞较少,平均为8.4%;滤泡辅助T细胞(T follicular helper cells,TFH)相关免疫组化标记阳性率分别为:CD10(7/14,50.0%)、BCL6(6/19,31.6%)、PD-1(13/19,68.4%)、CXCL13(13/19,68.4%),大部分病例肿瘤细胞PD-1和CXCL13同时阳性,且阳性细胞数量较少(均<1%)。24例行流式细胞术检测,其中22例均一致性表达胞质CD3(cCD3)、CD5、CD4、CD2,均不同程度表达CD10,部分病例胞膜CD3(sCD3)表达缺失(12/22,54.5%),部分病例CD7表达缺失(8/22,36.4%);2例未发现异常T细胞。基因重排:7例行T系基因重排检测,其中3例TCR重排阳性。结论AITL侵犯骨髓的肿瘤细胞比例较低且异型性小,容易漏诊及误诊,当骨髓中出现细胞成分较杂的淋巴细胞灶,提示AITL侵犯骨髓。流式细胞术发现表型异常T细胞(CD4和CD10双阳性),强烈提示AITL侵犯骨髓。AITL侵犯骨髓的诊断应综合考虑骨髓活检、流式细胞术及基因重排等检查结果。展开更多
Burkitt lymphoma is an aggressive lymphoma and its clinical, immunologic, chemical features are well-known. Burkitt lymphoma is in highgrade lymphomas and can spread very rapidly. Burkitt lymphoma has been difficult t...Burkitt lymphoma is an aggressive lymphoma and its clinical, immunologic, chemical features are well-known. Burkitt lymphoma is in highgrade lymphomas and can spread very rapidly. Burkitt lymphoma has been difficult to be cured with conventional chemotherapy for adults. Early autologous bone marrow should be kept in mind in first remission BL. We presented a case of presenting with rapid and multi organ involvement with BL. A 55-year-old male patient responded to the conventional chemotherapy for short-term and died 9 months after diagnosis because of relapse of Burkitt lymphoma.展开更多
基金Supported by National Science and Technology Major Subproject of China,No.2018ZX10302205-002Chinese Foundation for Hepatitis Prevention and Control-Tianqing Liver Disease Research Fund Subject,No.TQGB2020168.
文摘BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.
文摘Objective: Primary lymphoma of bone (PLB) is a rare malignant bone tumor often presenting in the fifth-sixth decades involving appendicular long bones. Published radiological findings indicate that PLB typically presents as a moth-eaten osteolytic lesion with periosteal reaction, while MRI commonly demonstrates marrow infiltration with extraosseous extension. Given rarity and variable appearances, PLB may not be primarily considered prior to biopsy. Our objective was to evaluate preoperative imaging findings in effort to increase awareness and improve a perceived deficiency in preoperative diagnosis. Materials and Methods: Following IRB approval, retrospective review identified 60 patients with newly diagnosed bone lesions proven to represent PLB in accordance with WHO definition. Preoperative radiographs (n = 46), MRI (n = 33) and PET (n = 37) were independently reviewed by two radiologists. At radiography, lesions were classified: purely lytic, mixed, purely sclerotic, or occult;lytic lesions were graded utilizing Lodwick’s classification. At MRI, lesions were defined as focal or infiltrative and the presence or absence of extraosseous disease was recorded. Extraosseous masses were defined as small (1 cm) and subjectively correlated with degree of cortical destruction. At PET, lesions were recorded as FDG-avid or not. Primary radiograph reports when available (n = 33) were reviewed and exact wording of differential considerations was recorded. Results: Radiographs demonstrated mixed (n = 22), lytic (n = 15), and sclerotic (n = 8) appearances;one radiographically occult lesion was seen by MRI. Lytic lesions were graded: IB (n = 3), IC (n = 5), II (n = 4), and III (n = 3);none were IA. At MRI, 30 lesions were infiltrative and 3 were focal;11 were not associated with extraosseous extension, while 22 showed bony disease with small (n = 7) or large (n = 15) soft tissue mass. Of large masses, 13 demonstrated minimal cortical destruction. At PET, 36 demonstrated FDG uptake;one study was technically limited. Review of reports found that only 5 included “lymphoma” as a diagnostic consideration. Conclusion: Contrary to most published data, we suggest that PLB typically demonstrates some degree of osteosclerosis, often a mixed pattern of sclerosis and lucency;purely lytic lesions may be less common. Similar to existing reports, MRI commonly demonstrates marrow infiltration with extraosseous extension of disease, typically a large soft tissue mass with disproportionate (minimal) cortical destruction. Familiarity with these findings should improve preoperative consideration of PLB in the appropriate clinical scenario when a new osteoblastic lesion is identified.
文摘Primary mediastinal neuroendocrine tumors are a rare malignancy that accounts for < 10% of all mediastinal tumors. The case presented here involves a 52-yearold man who had been suffering for 3 mo from chronic cough, anorexia and substantial weight loss, as well as 2 wk of jaundice prior to his admission. A computed tomography scan showed a 4.3 cm × 6.6 cm mediastinal mass with multiple liver nodules scattered along both hepatic lobes. Endoscopic ultrasound showed a large heterogeneous hypoechoic mass at the mediastinum with multiple target-like nodules in the liver. Fine-needle aspiration specimens revealed numerous, small, round cells with hyperchromatic nuclei, scarce cytoplasm, and frequent mitotic features. Immunohistochemical study revealed positive results for AE1/AE3, CD56 and chromogranin A, with negative findings for synaptophysin, CK20, vimentin, CK8/18 and CD45. The patient was subsequently diagnosed with a poorly differentiated neuroendocrine carcinoma, small cell type. A bone marrow biopsy also revealed extensive involvement by the carcinoma.
文摘Primary non-Hodgkin’s lymphoma of the bone (PLB) is extremely rare in the pediatric population with less than 100 cases reported in the English literature. Most commonly, patients present with atraumatic bone pain and grossly normal radiographic findings. PLB is in the histopathological class of “small round cell tumors of bone”, as with most common bone tumors. The diagnosis is confirmed by immunohistochemical or flow cytometry based detection of tumor-specific proteins. We present a case of stage IV PLB of B-lymphoblastic type with an excellent response to chemotherapy to increase awareness among general pediatricians and pathologists about the importance of making the correct diagnosis, given the excellent prognosis for this disease.
文摘Introduction: Diffuse large B-cell lymphoma (DLBCL), not otherwise specified, is a large B-cell lymphoma with a diffuse growth pattern and aggressive clinical course. It is divided in subgroups according to its morphology, immunophenotype, and primary site. Dissemination to bone marrow occurs in 11% to 35% of cases and can be of concordant or discordant morphology. Objective: To examine the association, the type of bone marrow involvement in relation to the primary site, morphology, immunohistochemistry of DLBCLs and to determine the cases of Epstein-Barr virus positive DLBCLs. Materials and Methods: We reviewed lymph node and extranodal biopsies as well as the respective bone marrow biopsies in all cases of DLBCL diagnosed in the Hospital General de México during the period from 2002 to 2010. We used immunohystochemistry for immunophenotype identification (Hans’s algorithm) and an in-situ hybridization technique to detect presence of Epstein Barr encoded RNA (EBER). Results: We included 108 patients with a mean age of 51.9 years, 59 (55%) were men. DLBCL involved lymph nodes in 60% of cases and palatine tonsils in 13%. The centroblastic variant predominated (80%) and 58% originated from activated B-cells. Infiltration of bone marrow was present in 30% of cases and was discordant in 55% of these cases. Correlation between morphology and bone marrow infiltration was statistically significant (P = 0.0003). Presence of Epstein-Barr virus was demonstrated in 15% of patients older than 50 years. Conclusions: Dissemination to bone marrow occurred in 30% of cases and discordant involvement was most common. DLBCL originating from activated B-lymphocytes predominated and the most common extranodal sites were palatine tonsils, suggesting that our population has a clinical behavior similar to Asiatic populations.
文摘Burkitt lymphoma is an aggressive lymphoma and its clinical, immunologic, chemical features are well-known. Burkitt lymphoma is in highgrade lymphomas and can spread very rapidly. Burkitt lymphoma has been difficult to be cured with conventional chemotherapy for adults. Early autologous bone marrow should be kept in mind in first remission BL. We presented a case of presenting with rapid and multi organ involvement with BL. A 55-year-old male patient responded to the conventional chemotherapy for short-term and died 9 months after diagnosis because of relapse of Burkitt lymphoma.
